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Adapted image from Montenovo MI, Jalikis FG, Hoch B, Bakthavatsalam R. Case Rep Transplant. 2014;2014:934385. [Open access.] PMID: 25580347, PMCID: PMC4279711.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

Endocrine emergencies comprise several relatively uncommon but potentially life-threatening conditions that can be diagnostic and management challenges, leading to delays in timely recognition and treatment. Familiarize yourself with correctly identifying and appropriately addressing the following 12 endocrine emergencies.

This MIBG (iodine-123-meta-iodobenzylguanidine) scan shows avid uptake of radiotracer in the right adrenal gland of an elderly patient with symptomatic de novo pheochromocytoma.

Adapted images from Patel P, Bekkerman M, Varallo-Rodriguez C, Rampersaud R. Case Rep Crit Care. 2016;2016:3438080. [Open access.] PMID: 27872766, PMCID: PMC5107213.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

An elderly woman presented with a nonconvulsive seizure and myxedema coma complicated by a right middle cerebral artery infarct. Top row: Electroencephalograms (EEGs) on admission (left) and 10 days later (right). Vertical arrows = paroxysmal discharges. Bottom row: Head computed tomography (CT) scans on admission (left) and 14 days later (right).

Myxedema Coma

Myxedema coma is a rare but life-threatening endocrine emergency commonly seen in patients with a history of hypothyroidism—occasionally, it may be the initial presenting symptom/sign. Women are predominantly affected,[1] and it is almost always seen in those older than 60 years.[2]

Myxedema coma is typically triggered by an inciting event such as an infection, gastrointestinal (GI) bleed, trauma, initiation of new medication, or hypoglycemia. Symptoms/signs include altered mental status, decreased respiratory rate, hypothermia, hypotension, bradycardia, hyponatremia, and hypoglycemia.[2]

Adapted images from Takamura A, Sangen R, Furumura Y, Usuda D, Kasamaki Y, Kanda T. Clin Case Rep. 2017;5(4):399-402. [Open access.] PMID: 28396755, PMCID: PMC5378864.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

These are admission images from an elderly woman with myxedema coma who presented with new-onset severe hypothyroidism and renal failure. Top left (thyroid sonogram): Normal-sized thyroid with small nodules. Bottom left (electrocardiogram [ECG]): Low-voltage waves throughout. Right (chest x-ray): Cardiomegaly and pleural effusion.

Clinicians should assess for dry skin, thin hair, nonpitting edema, periorbital edema, ptosis, and hypoactive bowel sounds.[2,3] The presence of an anterior neck scar hints at the possibility of a previous thyroidectomy. The ECG may show low voltages, and chest radiography can reveal a large cardiac silhouette.

In patients with suspected myxedema coma, blood studies should include levels of thyroid-stimulating hormone (TSH), free thyroxine (T4), triiodothyronine (T3), and cortisol.[4] After the tests are drawn, initiate intravenous (IV) thyroid hormone replacement with T4 and/or T3. No randomized controlled trials exist to determine if T3 or T4 replacement is superior; however, the American Thyroid Association (ATA) recommends combination therapy with both.[4] If concomitant adrenal insufficiency is suspected, steroids should be given.[1]

Left: Photo showing exophthalmos from Wikimedia Commons / vasser a. Right: CT scan of enlarged thyroid gland courtesy of Nicole Cimino-Fiallos, MD, FAAEM.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

Thyroid Storm

Thyroid storm occurs when excessive release of thyroid hormone occurs.[3,5,6] Hyperthermia, hypertension, tachycardia, diaphoresis, and altered mental status may be seen in a patient with a history or signs of hyperthyroidism (eg, thinning hair, exophthalmos, goiter). Symptoms/signs of high-output heart failure may be present.[3,5,6]

Laboratory work-up should include measurements of TSH, free T3, and T4.[3,5,6] Reducing the sympathetic surge in these patients is critical. IV beta blockers (ideally propranolol) should be initiated to lower the heart rate to below 100 beats/minute, followed by IV administration of the antithyroid agent propylthiouracil (PTU) or methimazole to block thyroid hormone synthesis. After 1 hour, give iodine orally or via nasogastric tube to block release of thyroid hormones. Consider administering steroids to decrease T4 conversion to T3.[3,5,6] Finally, patients with thyroid storm are hyperthermic; although passive cooling is encouraged, active cooling can precipitate peripheral vasoconstriction and worsen hypertension.[5]

Sonograms of Hashimoto thyroiditis from Cappelli C, Pirola I, Gandossi E, Formenti A, Agosti B, Castellano M. Int J Endocrinol. 2015;2015:367054. [Open access.] PMID: 26273296, PMCID: PMC4530237. Micrograph of subacute thyroiditis from Wikimedia Commons / Nephron.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

Thyroiditis

Thyroiditis may be difficult to diagnose because it can present with symptoms/signs of either hypo- or hyperthyroidism, depending on the etiology. If thyroid cells are destroyed quickly, large boluses of thyroid hormone are released into the circulation and cause thyrotoxicosis.[7] If thyroid cells are damaged over time, symptoms/signs of a hypoactive thyroid will develop.

Several different types of thyroiditis exist, including Hashimoto, postpartum, postradiation, Riedel, and de Quervain.[7] Many of these types are related to autoimmune disease. Thyroiditis is most common in women and peaks in middle age.[8] Some drugs can also cause thyroiditis, including lithium and amiodarone.[8] The diagnosis of thyroiditis often hinges on findings from the physical examination and laboratory testing, including levels of thyroglobulin and TSH, and erythrocyte sedimentation rate. Treatment is dependent on the type of thyroiditis.[7]

Image from Naka N, Takenaka S, Nanno K, et al. World J Surg Oncol. 2007;5:27. [Open access.] PMID: 17338824, PMCID: PMC1821329.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

A patient suffered acute adrenal crisis after orthopedic surgery for a pathologic fracture. This CT scan depicts bilateral adrenal enlargement consistent with lung cancer metastases (asterisks).

Adrenal Crisis

Adrenal crisis may develop from primary adrenal insufficiency (caused by autoimmunity, metastatic disease, or ischemic or hemorrhagic lesions) or secondary adrenal insufficiency (caused by panhypopituitarism or pituitary corticotropic insufficiency).[3] However, the most common at-risk patients are those taking chronic steroids who develop adrenal suppression over time and then undergo abrupt steroid withdrawal.[3] In patients with sepsis and coagulopathy, Waterhouse–Friderichsen syndrome and hemorrhagic adrenalitis can also cause adrenal crisis.[9]

Image from Khwaja J. J Med Case Rep. 2017;11(1):72. [Open access.] PMID: 28302165, PMCID: PMC5356297.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

A patient presented with Escherichia coli sepsis and adrenal crisis. This abdominal-pelvis CT scan with contrast shows bilateral adrenal hemorrhages (arrows).

Adrenal crisis should also be considered in cancer patients taking ipilimumab or nivolumab, as these agents cause adrenalitis.[10] Alternatively, medications such as antiepileptics, barbiturates, and antifungals can also interact with steroid metabolism and cause acute adrenal insufficiency.[9]

Adrenal crisis can be defined as (1) including at least two of the following symptoms: hypotension, nausea/vomiting, fatigue, hyponatremia, hypoglycemia, and hyperkalemia; and (2) it improves after parenteral administration of glucocorticoids.[11] Treatment of adrenal crisis consists of 100 mg IV hydrocortisone and IV fluid resuscitation, as well as management of the precipitating factors.[3,11]

Adapted images from (left) Aziz Siddiqui S, Soomro N, Ganatra A. Pak J Med Sci. 2013;29(1):220-3. [Open access.] PMID: 24353545, PMCID: PMC3809185; and (right) Raul G Palacios III, MD, Brooke Army Medical Center, via MedPix [public domain].

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

Images from two patients with delayed presentation of congenital adrenal hyperplasia (CAH). Left: Clitoromegaly with normal labia and introitus. Right: CT scan with contrast through the adrenals showing marked symmetric enlargement of the adrenals and no other significant abnormality.

Congenital Adrenal Hyperplasia

CAH is the most common form of pediatric primary adrenal insufficiency.[12] Females become virilized in utero and typically present at birth with ambiguous genitalia, but males can be more difficult to diagnose. Although most affected male patients are detected on newborn screening, those who do not undergo such screening are at high risk for serious complications and may present to the emergency department (ED) with symptoms/signs of salt wasting, which includes lethargy, poor tone, vomiting, poor feeding, or failure to thrive.[12] Clinical manifestations typically appear around age 2-3 weeks in these male infants.

Patients with CAH should be treated with oral hydrocortisone for glucocorticoid replacement.[12]

Adapted images from Dolan RT, Butler JS, Mentee GP, Byrne M. J Med Case Rep. 2011;5:6. [Open access.] PMID: 21226896, PMCID: PMC3025966.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

These images were obtained in a patient with a right-side pheochromocytoma that presented as recurrent urinary tract infections. Top left (sonogram): Note the large, mixed cystic/solid adrenal mass. Top right (CT scan): A solid heterogeneous right adrenal mass is seen with focal cystic degeneration and slight displacement of the right kidney. Bottom center: The postsurgical gross adrenal pheochromocytoma specimen.

Pheochromocytoma

Pheochromocytomas are neuroendocrine tumors that develop from the chromaffin cells of the adrenal medulla and secrete norepinephrine and epinephrine.[13,14] Left undiagnosed and/or untreated, these lesions can precipitate life-threatening hypertension or cardiac arrhythmias.[14] Patients with pheochromocytomas classically present with paroxysmal hypertension, sweating, headache, and palpitations.[13,14]

Adapted images from Montenovo MI, Jalikis FG, Hoch B, Bakthavatsalam R. Case Rep Transplant. 2014;2014:934385. [Open access.] PMID: 25580347, PMCID: PMC4279711.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

These are the gross (left) and histologic (hematoxylin-eosin stain, 20× magnification) (right) images of the pheochromocytoma specimen from the same patient discussed on slide 1.

The gold standard for diagnosing pheochromocytomas is to measure the 24-hour urinary metanephrines (metabolites of norepinephrine and epinephrine).[13,14] Treatment of these tumors involves emergent surgical resection; if the tumor is not excised, patients can progress to pheochromocytoma multisystem crisis, which includes multiorgan failure, severe hypertension, hyperthermia, and encephalopathy.[13]

Preoperative preparation should include treating patients with an alpha blocker, such as doxazosin or prazosin.[14,15] Although hypertension must be controlled, a nonspecific beta-adrenergic receptor (eg, propranolol) should never be used.

Adapted images from Makdsi F, Kolade VO. Cases J. 2009;2:8095. [Open access.] PMID: 19918451, PMCID: PMC2769401.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

The chest x-ray (left) shows subcutaneous emphysema (top arrow) and pneumomediastinum at the cardiac border (bottom arrow), findings that were confirmed on CT scan (right), in a patient with diabetic ketoacidosis (DKA).

Diabetic Ketoacidosis

DKA is a dangerous complication of diabetes defined by a triad of hyperglycemia, acidosis, and ketonuria or ketonemia.[16,17] Triggers that can cause the cascade of DKA include infection, steroid therapy, trauma, and medication noncompliance.

The first step in treatment is to replace fluid volume, as these patients are extremely hypovolemic.[16,17] A rapid IV bolus of crystalloid fluid should be started (20 mL/kg in the first hour). Calculation of the total body water deficit may be helpful. In children, fluid resuscitation should be carefully titrated to avoid cerebral edema, whereas adults can undergo a more rapid fluid resuscitation.

After an initial fluid bolus, an insulin infusion may be started, with the goal of reducing ketone production and correcting the acidosis.[16,17] Exogenous glucose via 5% or 10% dextrose (D5 or D10) infusion may be required if patients become hypoglycemic while on insulin yet remain acidotic. Closely monitor potassium and magnesium levels, as insulin will shift these electrolytes.[16,17]

Adapted image from John S, Samuel S, Lakhan SE. J Med Case Rep. 2013;7:51. [Open access.] PMID: 23432798, PMCID: PMC3599740.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

Axial brain CT scans (A) and magnetic resonance images (MRIs) (B-D) of a patient with diabetic hyperglycemic hyperosmolar syndrome (HHS). The CT scans show diffuse leukoencephalopathy involving the frontal, parietal, and temporal lobes. The MRIs reveal extensive bilateral hemispheric white-matter abnormality.

Hyperglycemic Hyperosmolar Syndrome

HHS, another feared complication of uncontrolled diabetes, occurs less frequently than DKA but has a higher mortality.[18] It can also be triggered by infections, medication noncompliance, and trauma. The elderly, patients with type 2 diabetes or renal dysfunction, and those receiving steroids are more commonly affected. Clinicians should take note of polydipsia, polyuria, progressive dehydration, and weakness. HHS patients suffer from insulin resistance, resulting in increased gluconeogenesis, increased levels of cortisol and catecholamines, hyperglycemia, and osmotic diuresis. They make just enough insulin to prevent ketone production.[18]

Laboratory studies will reveal high osmolarity (>320 mOsmol/kg), hyperglycemia, and an elevated blood urea nitrogen (BUN)/creatinine ratio, but no ketoacidosis.[18] The anion gap and pH will most likely be in the normal range. Targeted treatment should include fluid volume replacement with crystalloids, initiation of insulin infusion, and correction of electrolyte imbalances. Complications of HHS include coma, seizures, shock, renal failure, rhabdomyolysis, thrombotic events, and fluid overload.[18]

Image from Abu-Abed Abdin A, Hamza M, Khan MS, Ahmed A. Case Rep Crit Care. 2016;2016:4275651. [Open access.] PMID: 27579186, PMCID: PMC4992786.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

This admission chest x-ray is from a diabetic patient with euglycemic DKA who was noncompliant with insulin and had cocaine intoxication.

Euglycemic DKA

Sodium-glucose cotransporter type 2 (SGLT2) inhibitors (eg, canagliflozin, dapagliflozin, empagliflozin) are a newer class of medications used to treat type 2 diabetes, but they can cause euglycemic DKA.[19] These drugs work by increasing the excretion of sugar in the patient's urine; when the amount of glucose in the blood falls with the use of SGLT2 inhibitors, insulin secretion may decline. Moreover, diabetic patients may choose to administer less insulin if their blood sugar is lower after starting on these agents. Lower levels of serum insulin and glucose can trigger the breakdown of free fatty acids, ketosis, and even ketoacidosis, despite the presence of a normal blood sugar level. Clinicians must be aware of this possible side effect, as the euglycemia can obscure the diagnosis of ketoacidosis, leading to a delay in treatment. Patients experiencing euglycemic DKA should be treated like any other patient in DKA.[19]

Adapted image from Albani A, Ferrau F, Angileri FF, et al. Int J Endocrinol. 2016;2016:7951536. [Open access.] PMID: 28074095, PMCID: PMC5198093.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

These coronal T1-weighted MRIs depict both intrasellar and suprasellar masses in two patients with pituitary apoplexy. Left: High peripheral signal intensity ring due to methemoglobin and slight compression of the optic chiasm (arrow). Right: Diffuse central high-signal intensity.

Pituitary Apoplexy

The pituitary gland plays a critical role in the control of the downstream endocrine glands, such as the adrenal glands and the thyroid. Apoplexy occurs when there is an acute hemorrhage or infarction of the pituitary gland, usually due to an adenoma.[20] Patients typically present with a sudden headache, vision changes, ophthalmoplegia, acute hypopituitarism, and altered mental status.

Adapted image from Albani A, Ferrau F, Angileri FF, et al. Int J Endocrinol. 2016;2016:7951536. [Open access.] PMID: 28074095, PMCID: PMC5198093.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

These coronal T2-weighted (A,B) and sagittal T1-weighted MRIs (C) in patients with pituitary apoplexy show (A) the low-signal hemorrhagic content of the pituitary mass, (B) the high-signal cystic area inside the lesion and the focal mucosal thickening of the sphenoid sinus (arrow), and (C) a fluid-fluid intralesional level (asterisk), which is pathognomonic of pituitary apoplexy.

The diagnostic test of choice for pituitary apoplexy is MRI, which can identify a hemorrhage or infarct inside the sella turcica.[20] Vision changes are due to compression on the optic chiasm, and ophthalmoplegia results from compression of cranial nerves 3, 4, and 6; surgical decompression may be required. The remainder of treatment is focused on replacing the lost hormones. Corticosteroid replacement is used to treat the secondary adrenal insufficiency that often results from pituitary apoplexy. In the immediate aftermath of illness, electrolyte levels should be carefully monitored and replaced. Over time, thyroid hormone replacement is also often necessary.[20]

ECG courtesy of Heather Murphy-Lavoie of Charity Hospital, New Orleans.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

Hypercalcemic Crisis

Severe hypercalcemia is defined as an albumin-corrected calcium level above 14 mg/dL.[21] This condition affects most organ systems and thus often displays multisystem dysfunction. Primary hyperparathyroidism is the leading cause of hypercalcemia in otherwise healthy patients.[21,22] They may complain of GI symptoms such as vomiting, abdominal pain, and constipation.

Findings on clinical examination and from laboratory studies may include signs of dehydration, renal colic, and acute kidney injury.[22] Some patients with hypercalcemia may present with confusion, poor concentration, or other nonspecific neurologic features. The ECG will likely exhibit a shortened QT interval (shown).[21,22]

Adapted image from Mageau A, Rigolet A, Benali K, et al. Medicine (Baltimore). 2016;95(10):e3089. [Open access.] PMID: 26962842, PMCID: PMC4998923.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

These are maximum-intensity projection, coronal positron emission tomography (PET) scans of a patient with life-threatening hypercalcemia and isolated sarcoidlike myositis who presented with abdominal pain. Left: Multiple linear and nodular intense fluorodeoxyglucose (FDG) uptake in the intercostal, back, shoulder, and thigh muscles. Right: Complete regression of the pathologic FDG uptake after 2 months of steroid treatment.

In general, hypercalcemia therapy depends on the calcium levels, clinical presentation, and cause.[23] The initial treatment of hypercalcemic crisis involves fluid volume correction with crystalloid solution and forced calciuresis.[21,23] Normal saline infusion will reduce calcium levels, but it will not return the patient to normocalcemia. Loop diuretics may also help in lowering calcium levels, as they block calcium reabsorption in the ascending limb of the loop of Henle.[23] Thiazide diuretics are contraindicated as they increase calcium levels.[22,23] IV bisphosphonates should be started after volume resuscitation has begun; these agents reduce serum calcium levels by inhibiting osteoclast activity.[21,23] However, most patients will ultimately require surgical parathyroidectomy.[22]

Image from Koufakis T, Gabranis I. Pan Afr Med J. 2014;17:65. [Open access.] PMID: 25018815, PMCID: PMC4085941.

On Strike or Working Overtime: 12 Endocrine Emergencies

Nicole Cimino-Fiallos, MD, FAAEM | July 12, 2019 | Contributor Information

Prolonged QT interval on ECG (left) and positive Trousseau sign (right) in a patient with hypocalcemia.

Acute Hypocalcemia

Acute hypocalcemia is most often caused by parathyroid gland dysfunction. A decrease in parathyroid hormone (PTH) results in increased excretion of calcium and decreased absorption. Acute hypocalcemia is frequently seen after thyroid surgery (if the parathyroid gland is iatrogenically removed) and manifests with carpopedal spasms, perioral numbness, extremity paresthesias, Chvostek sign (facial nerve tetany), and Trousseau sign (brachial artery occlusion causing flexion of the wrist and metacarpophalangeal joints, hyperextension of the fingers, and flexion of the thumb on the palm).[24] In severe cases, hypocalcemia can lower a patient's seizure threshold or potentially cause a cardiomyopathy.[24]

Those with symptomatic hypocalcemia should be considered for inpatient admission and treatment with IV calcium infusion.[24] Calcium gluconate is typically better tolerated than calcium chloride, which is more likely to cause a burning sensation at the infusion site. Continuous cardiac monitoring should be used during calcium administration, as rapid infusion can cause dysrhythmias. If hypomagnesemia is also present, it should be corrected before the hypocalcemia.[24] Finally, in patients with hypoparathyroidism-caused hypocalcemia that is refractory to first-line therapies, the US Food and Drug Administration has approved recombinant human PTH (1-84) for treatment.[24]

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Burning Up? A Guide to Treating Heat Illness

Heat illness can quickly become a medical emergency if not promptly recognized and treated. Do you know the best ways to cool down hyperthermic patients?Slideshows, June 2019
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Myxedema Coma or Crisis

Myxedema coma, occasionally called myxedema crisis, is a rare life-threatening clinical condition that represents severe hypothyroidism with physiological decompensation. The condition usually occurs in patients with long-standing, undiagnosed hypothyroidism and is usually precipitated by infection, cerebrovascular disease, heart failure, trauma, or drug therapy.Diseases/Conditions, October 2018
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References